1,721,022 research outputs found

    A MULTI-STATE MODEL EVALUATING THE ASSOCIATION OF OXYGEN THERAPY WITH THE COURSE OF CYSTIC FIBROSIS IN EUROPE

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    Introduction and Objective(s). The most serious complications of cystic fibrosis (CF) relate to respiratory failure, leading to hypoxemia, which is the time when the respiratory system is profoundly compromised by the disease. Oxygen therapy (OT) is then prescribed to restore oxygen levels in the blood.1 Also, some people with CF (pwCF) can have lung transplantation (LTx) during their life. Association between dependence OT and natural disease progression in pwCF becomes challenging, and it has not been estimated yet. We therefore used the multi-state model to estimate the transition probabilities from being alive without LTx to LTx and to death, and from being alive after LTx to death in pwCF with and without OT. Method(s) and Results. We used 10 years’ data from the 35-country European CF Society Patient Registry (ECFSPR). A multi-state regression model was fitted using age as timescale to assess the effects of individual risk factors on transition probabilities. We considered 48,343 pwCF aged 6 to 50 years. OT (HR 5.78, 95%CI: 5.32 - 6.29) and abnormal FEV1 (HR 6.41, 95%CI: 5.28 - 7.79) were strongly associated with the probability of having LTx; chronic infection with Burkholderia cepacia complex (HR 3.19, 95%CI: 2.78 - 3.67), abnormal FEV1 (HR 5.00, 95%CI: 4.11 - 6.08) and the need for OT (HR 4.32, 95%CI: 3.93 - 4.76) showed the greatest association with the probability of dying without LTx. Once pwCF received LTx, OT (HR 1.75, 95%CI: 1.41 - 2.16) and abnormal FEV1 (HR 1.63, 95%CI: 1.18 - 2.25) were the main factors associated with the probability of dying. We also found an association between gross national income and the probability of receiving LTx, which is lower for pwCF living in low-income European countries. Conclusions. Oxygen therapy, as a proxy for disease severity, is associated with poor survival in pwCF, even after LTx.; harmonization of CF care throughout European countries remains of paramount importance

    Ventilatory limitation and dynamic hyperinflation during exercise testing in Cystic Fibrosis

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    OBJECTIVE: To investigate the presence of dynamic hyperinflation after the Modified Shuttle Test (MST) and its relationship with lung function, exercise tolerance, and clinical symptoms in Cystic Fibrosis (CF). METHODS: Retrospective observational study. Subjects in clinically stable condition with a CF diagnosis based on a positive sweat test (chloride >60 mEq/L) and/or presence of two disease causing mutations, with available data on MST, spirometry, maximal voluntary ventilation, and inspiratory capacity manoeuvres were considered for the analysis. Breathing reserve was calculated and a threshold value of 0.7 was subsequently chosen as a value of pulmonary mechanical limit. Subjects were then categorized into two groups according to the change in the inspiratory capacity from rest to peak exercise. Unconditional logistic regression was used to estimate unadjusted odds ratios, 95% confidence intervals and P-values. RESULTS: Twenty-two subjects demonstrated evidence of dynamic hyperinflation during the MST. Thirteen out of 22 subjects were ventilatory limited during exercise including 5 of those without evidence of dynamic hyperinflation (P = 0.24). No combination of variables resulted in a parsimonious regression model. CONCLUSIONS: Dynamic hyperinflation is common in CF and it is not associated with traditionally defined ventilatory limitation parameters during the MST. Pediatr Pulmonol. 2017;52:29-33

    Aerosol delivery practice in Italian Cystic Fibrosis centres : a national survey

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    Background: Physiotherapists (PTs) are ideally positioned to assist patients and families with inhalation therapies through monitoring, communication and education about available therapies and their proper use; indeed aerosoltherapy management is listed as part of Italian PTs' core competence and in the core syllabus for post-graduate training in respiratory physiotherapy. The aim of this study was to outline the involvement of Italian PTs working in Cystic Fibrosis (CF) centres in the aerosol delivery practice. Methods: Physiotherapist coordinators (n = 29) of all Italian CF centres were invited to participate in a cross-sectional survey and a semi-structured questionnaire was developed and sent by e-mail. Results: A response rate of 69 % was achieved. The majority of participants were woman and the overall mean professional experience was twenty years. Italian PTs are involved in the aerosol delivery practice, managing education, drug-device combination, dilution and mixing of drugs. Conclusions: Physiotherapists play a key role in the care of Italian CF patients; nevertheless the Italian Group of Physiotherapists might plan interventions to harmonize the aerosol delivery practice in Italian CF centres and to sustain continuing education

    Going Beyond Counting First Authors in Author Co-citation Analysis

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    The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed

    Variations on the Author

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    “Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
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